모아모야병 연관 두통 환자에서 뇌 자기공명혈관조영 소견의 악화에 따른 두통 형태의 변화 전남대학교 의과대학 소아과학 교실1, 영상의학교실2, 조선대학교 의과대학 소아과학교실3 이우진1・김영옥1・윤 웅2・노영일3・우영종1
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Case report J Korean Child Neurol Soc 2014;22(2):98-101 pISSN 1226-6884 모아모야병 연관 두통 환자에서 뇌 자기공명혈관조영 소견의 악화에 따른 두통 형태의 변화 전남대학교 의과대학 소아과학 교실1, 영상의학교실2, 조선대학교 의과대학 소아과학교실3 이우진1・김영옥1・윤 웅2・노영일3・우영종1 Headache Associated with Moyamoya Disease in a Woo Jin Lee, MD1, Young Ok Kim, MD1, Woong Child: Pain Pattern in MRA Progression Yoon, M.D2, Young Il Rho, M.D3 Young Jong Woo, MD Headache associated with moyamoya disease (HAMD) has been reported in about 20–30% of pediatric cases with moyamoya disease. However, the characteristic Department of Pediatrics1 and Radiology2, Chonnam patterns of HAMD during disease progression are not completely known, although National University Medical School, and Department much is known on the incidence of HAMD and on the treatment effects of bypass of Pediatrics3, School of Medicine, Chosun University, surgery. In a child who presented with HAMD and had no infarction or hemorrhage Gwangju, Republic of Korea till bypass surgery, we described the characteristic patterns of headache progression. At first presentation, the patient complained of severe bilateral headache despite mild stenosis only in the left internal carotid artery on brain magnetic resonance angiography. During all stages of progression, headache was severe and pressing in nature rather than throbbing. Nausea and vomiting were present even in the early stage, but photophobia, phonophobia and neck stiffness developed in the late stage. Headache was aggravated by menstruation and was relieved by analgesics and to- piramate in the early stage, although the medication failed to provide relief when the headache increased in frequency and duration. Key Words: Moyamoya disease, Headache, Child Introduction Moyamoya disease (MD), a slowly progressive cerebrovascular occlusive disease of the distal internal carotid and cerebral arteries, is characteristically accompanied with puff-like collateral vessels at the base of the brain1-4). According to the report of Submitted: 21 May, 2014 Revised: 20 June, 2014 Kim et al., headache was observed at presentation in 33% of 410 patients with Accepted: 28 June, 2014 pediatric MD5). Headache was the third most common symptoms in pediatric MD following transient ischemic attacks (55.9%) and infarction (39%)5). In their study, Correspondence to Young Ok Kim, MD, PhD Department of Pediatrics, Chonnam National University the Headache associated with moyamoya disease (HAMD) was defined as severe Medical School, 671, Jebongro, Dong-gu, Gwangju, headache, that disturbed daily activity, required rest or medication, and occurred at 501-757, Republic of Korea. least once a month5). Seol et al. also detected HAMD in 21.6% of 204 pediatric Tel +82-62-220-6646, Fax: +82-62-220-6646 E-mail [email protected] patients with MD before surgery6). They observed nausea or vomiting in 27.3% of patients with HAMD, eyeball pain in 6.8% and ab dominal pain in 4.5%6). Headache was localized only in 11.4% of patients and was provoked in 9.1% by hyperventilation6). Although HAMD has been commonly re ported by these large Copyright © 2014 by The Korean Child Neurology Society http://www.cns.or.kr http://www.cns.or.kr J Korean Child Neurol Soc 2014;22(2):98-101 99 studies for pediatric MD, the characteristics and classification of Headaches were associated with dizziness, weakness or numb- HAMD are still rarely known5,6). Description of headache pro- ness in the right upper extremity and ipsilateral side of face. gression based on a headache diary is rare, and clinical charac- At about 13 years of age, the patient developed a severe head- teristics of HAMD have been more precisely described only in ache in the early morning with nausea and vomiting. The second several case reports7-12). Herein, we described progressive clinical MRA showed additional stenosis in the right distal ICA, and MCA patterns of headache in a child with HAMD at Suzuki stage 1 to in addition to previous mild stenosis in the left distal ICA and MCA 3 on brain magnetic resonance angiography (MRA). without infarction on brain magnetic resonance imaging (MRI) (Fig. 1B). [99mTc]-ECD brain single photon emission computed tomography showed a perfusion defect in the right parietal and Case report anterior temporal lobes and a decreased vascular reserve in the right parietal, temporal, frontal and left temporal lobes after ace- A 12-year-old, previously healthy girl developed a sudden, tazolamide administration. Bypass surgery was recommended continuous, severe, pressing, bi-temporal headache. The headache and topiramate (50 mg/day) treatment ameliorated the headaches. was followed by dysarthria, weakness in right hand, and numbness During follow-up up to about 15 years of age, while awaiting in right hand and the ipsilateral side of the face. There was no bypass surgery in another hospital, the patient developed frequent family history of migraine or cerebro-vascular accidents. About headaches followed by transient weakness in her left limbs and a year earlier, she had suffered a similar severe headache, twice ipsilateral eyeball pain. The third MRA at 14 years and 6 months (Table 1). In the first episode, eyeball pain was predominant but of age, showed severe stenosis in the right distal ICA and MCA, an ophthalmologic evaluation showed no abnormality (Table 1). near occlusive stenosis in the left distal ICA and MCA (Fig. 1C), On the first visit to our hospital, brain MRA showed mild stenosis and rich basal collateral vessels (Fig. 1D), without ischemic or in the left distal internal carotid artery (ICA) and M1 portion of hemorrhagic infarction on brain MRI. Headaches were still ag- the left middle cerebral artery (MCA) (Fig. 1A). Similar attacks gravated by menstruation. The frequency of headaches increased occurred 4 more times over the next year. Headaches were us- to 10 times a year and neither NSAID nor preventive topiramate ually bi-temporal, persisting nearly all day long, and aggravated treatment (100 mg/day) was effective in ameliorating the symptoms. during the menstrual period (Table 1). It was occasionally con- trolled by non-steroidal anti-inflammatory drugs (NSAID) or rest. Table 1. Characteristics of Headache Associated with Moyamoya Disease in Each Progressive Stage of Magnetic Resonance Angiography 0 month 2 months (2nd 15 months (3rd attack, -26 months -44 months After symptom onset (1st attack) attack) 1st brain image) (2nd brain image) (3rd brain image) Age 10Y9M 10Y11M 12Y (1st visit) 12Y11M 14Y5M Brain MRA stage* in right/left 0 / 1 1/1 3/3 Transient ischemic attack No Yes Yes Yes Yes Weakness - Right upper Right upper Right upper & lower Left upper & lower, Left facial Numbness - Right upper & Right upper & Right upper & - Right facial Right facial Right facial Dysarthria - + + - - Headache Localization - Obscurely bilateral Both temporal Left/right frontal or Both Left temporal or Left temporal, Shoulder & neck occipital Frontal Propagation Eyeball pain +, both - - - +, left Quality Pressing Pressing Pressing Pressing or throbbing Pressing Severity Severe Severe Severe Severe Severe Duration < 12 hr < 12 hr 12~24 hr 12~24 hr 12-24 hr Frequency (/year) 3 4 10 *Probable Suzuki stage on brain magnetic resonance angiography (MRA) is based on MRA generated progressive appearance of moyamoya disease, adapted from angiographic findings from stages one through six, as originally defined by Suzuki and Takaku in 19691). Y, year; M, month; MRA, magnetic resonance angiography. 100 Lee WJ, et al. • Headache with Moyamoya Disease http://www.cns.or.kr Discussion morrhagic or ischemic) and to which Suzuki stage it belongs. Clinical feature of HAMD may largely differ between pediatric The mechanism underlying HAMD has been suggested to be and adult patients, as the common clinical findings in childhood multifactorial, including a role for cerebral artery nociceptors, MD are recurrent ischemic attacks as opposed to adult presentation the effect of an aneurysm resulting in stimulation of trigeminal of intracranial hemorrhage1-4). The Suzuki stage (1-6) is associated nociceptors or bleeding into the subarachnoid space, neurogenic with progressive angiographic appearance of MD, as originally inflammation, and cerebral hypoperfusion7). Although the causal defined by Suzuki and Takaku in 1969 (Table 2)1). Suzuki et al. mechanism for HAMD is still not clearly known, vascular pathology, reported that the degree of change in collateral flow according such as progressive occlusion of intracranial arteries or aneurysm to the stages was only apparent in children but was not in adults1). formation in MD, is suspected as the main eitiology6,7). The nature The apparent collateral flow in children was considered because of HAMD can be presumably different based on the type (he- they have a greater ability to form collateral vessels than adults1). Fig. 1. Series of brain magnetic resonance angiography (MRA) images. (A) Initial brain MRA showed mild stenosis (arrow) in the M1 portion of the left middle cerebral artery (MCA). (B) The second brain MRA obtained a year later showed no change in mild stenosis in the M1 of left MCA. MRA also revealed newly seen, diffuse moderate stenosis (short arrows) in the distal supraclinoid portion of right internal carotid artery (ICA) and M1 of the right MCA, and focal severe stenosis (long arrow) at the site of origin of the superior division of the right MCA. (C, D) Follow-up MRA obtained 19 months after the second MRA showed the typical appearance of advanced moyamoya disease; severe stenosis in both the right distal ICA and the right MCA,